{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T11:35:33Z","timestamp":1791286533110,"version":"4.1.0"},"reference-count":153,"publisher":"Elsevier BV","issue":"10","license":[{"start":{"date-parts":[[2011,10,1]],"date-time":"2011-10-01T00:00:00Z","timestamp":1317427200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2011,10,1]],"date-time":"2011-10-01T00:00:00Z","timestamp":1317427200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2013,7,18]],"date-time":"2013-07-18T00:00:00Z","timestamp":1374105600000},"content-version":"vor","delay-in-days":656,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"},{"start":{"date-parts":[[2011,10,1]],"date-time":"2011-10-01T00:00:00Z","timestamp":1317427200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2011,10,1]],"date-time":"2011-10-01T00:00:00Z","timestamp":1317427200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2011,10,1]],"date-time":"2011-10-01T00:00:00Z","timestamp":1317427200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2011,10,1]],"date-time":"2011-10-01T00:00:00Z","timestamp":1317427200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2011,10,1]],"date-time":"2011-10-01T00:00:00Z","timestamp":1317427200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["DK-57328"],"award-info":[{"award-number":["DK-57328"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000005","name":"U.S. Department of Defense","doi-asserted-by":"publisher","award":["CDMRP PR093488"],"award-info":[{"award-number":["CDMRP PR093488"]}],"id":[{"id":"10.13039\/100000005","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"published-print":{"date-parts":[[2011,10]]},"DOI":"10.1016\/j.bbadis.2010.11.014","type":"journal-article","created":{"date-parts":[[2010,12,12]],"date-time":"2010-12-12T04:04:58Z","timestamp":1292126698000},"page":"1337-1343","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":63,"title":["Polycystic kidney disease: Pathogenesis and potential therapies"],"prefix":"10.1016","volume":"1812","author":[{"given":"Vinita","family":"Takiar","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J.","family":"Caplan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.bbadis.2010.11.014_bb0005","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1038\/ki.2009.128","article-title":"Autosomal dominant polycystic kidney disease: the last 3years","volume":"76","author":"Torres","year":"2009","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0010","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.1016\/S0140-6736(07)60601-1","article-title":"Autosomal dominant polycystic kidney disease","volume":"369","author":"Torres","year":"2007","journal-title":"Lancet"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0015","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1056\/NEJMra022161","article-title":"Polycystic kidney disease","volume":"350","author":"Wilson","year":"2004","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0020","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1111\/j.1600-6143.2007.02030.x","article-title":"Mosaicism in autosomal dominant polycystic kidney disease revealed by genetic testing to enable living related renal transplantation","volume":"8","author":"Connor","year":"2008","journal-title":"Am. J. Transplant."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0025","doi-asserted-by":"crossref","first-page":"1468","DOI":"10.1038\/ki.2008.485","article-title":"Characterization of large rearrangements in autosomal dominant polycystic kidney disease and the PKD1\/TSC2 contiguous gene syndrome","volume":"74","author":"Consugar","year":"2008","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0030","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1038\/ki.1997.373","article-title":"Association of the angiotensin I converting enzyme gene deletion polymorphism with early onset of ESRF in PKD1 adult polycystic kidney disease","volume":"52","author":"Baboolal","year":"1997","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0035","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1016\/S0272-6386(98)70072-1","article-title":"Cystic fibrosis and the phenotypic expression of autosomal dominant polycystic kidney disease","volume":"32","author":"O'Sullivan","year":"1998","journal-title":"Am. J. Kidney Dis."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0040","doi-asserted-by":"crossref","first-page":"2285","DOI":"10.1681\/ASN.V11122285","article-title":"CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease","volume":"11","author":"Persu","year":"2000","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0045","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1038\/ng1294-328","article-title":"Deletion of the TSC2 and PKD1 genes associated with severe infantile polycystic kidney disease\u2014a contiguous gene syndrome","volume":"8","author":"Brook-Carter","year":"1994","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0050","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1038\/ki.1992.195","article-title":"Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease","volume":"41","author":"Gabow","year":"1992","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0055","doi-asserted-by":"crossref","first-page":"1349","DOI":"10.1681\/ASN.V561349","article-title":"Overt proteinuria and microalbuminuria in autosomal dominant polycystic kidney disease","volume":"5","author":"Chapman","year":"1994","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0060","doi-asserted-by":"crossref","first-page":"1654","DOI":"10.1046\/j.1523-1755.2001.0590051654.x","article-title":"Progression of autosomal-dominant polycystic kidney disease in children","volume":"59","author":"Fick-Brosnahan","year":"2001","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0065","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.1053\/ajkd.2002.33379","article-title":"Relationship between renal volume growth and renal function in autosomal dominant polycystic kidney disease: a longitudinal study","volume":"39","author":"Fick-Brosnahan","year":"2002","journal-title":"Am. J. Kidney Dis."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0070","doi-asserted-by":"crossref","first-page":"1177","DOI":"10.1038\/ki.1990.330","article-title":"Renal structure and hypertension in autosomal dominant polycystic kidney disease","volume":"38","author":"Gabow","year":"1990","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0075","doi-asserted-by":"crossref","first-page":"1792","DOI":"10.1046\/j.1523-1755.2003.00264.x","article-title":"The role of parental hypertension in the frequency and age of diagnosis of hypertension in offspring with autosomal-dominant polycystic kidney disease","volume":"64","author":"Schrier","year":"2003","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0080","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1681\/ASN.V81105","article-title":"Utility of ultrasonography in the diagnosis of autosomal dominant polycystic kidney disease in children","volume":"8","author":"Gabow","year":"1997","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0085","doi-asserted-by":"crossref","first-page":"148","DOI":"10.2215\/CJN.00330705","article-title":"Volume progression in autosomal dominant polycystic kidney disease: the major factor determining clinical outcomes","volume":"1","author":"Grantham","year":"2006","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0090","doi-asserted-by":"crossref","first-page":"2214","DOI":"10.1046\/j.1523-1755.2003.00326.x","article-title":"Magnetic resonance measurements of renal blood flow as a marker of disease severity in autosomal-dominant polycystic kidney disease","volume":"64","author":"King","year":"2003","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0095","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1046\/j.1523-1755.2003.00185.x","article-title":"Renal structure in early autosomal-dominant polycystic kidney disease (ADPKD): The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) cohort","volume":"64","author":"Chapman","year":"2003","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0100","doi-asserted-by":"crossref","first-page":"2122","DOI":"10.1056\/NEJMoa054341","article-title":"Volume progression in polycystic kidney disease","volume":"354","author":"Grantham","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0105","doi-asserted-by":"crossref","first-page":"719","DOI":"10.2215\/CJN.03750708","article-title":"MRI-based kidney volume measurements in ADPKD: reliability and effect of gadolinium enhancement","volume":"4","author":"Bae","year":"2009","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0110","doi-asserted-by":"crossref","first-page":"1058","DOI":"10.1053\/j.ajkd.2005.08.026","article-title":"Sonographic assessment of the severity and progression of autosomal dominant polycystic kidney disease: the Consortium of Renal Imaging Studies in Polycystic Kidney Disease (CRISP)","volume":"46","author":"O'Neill","year":"2005","journal-title":"Am. J. Kidney Dis."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0115","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1080\/08860220601038892","article-title":"Improvement of endothelial dysfunction with simvastatin in patients with autosomal dominant polycystic kidney disease","volume":"29","author":"Namli","year":"2007","journal-title":"Ren. Fail."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0120","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1016\/S0022-5347(05)67276-X","article-title":"Bilateral nephrectomy with concomitant renal graft transplantation for autosomal dominant polycystic kidney disease","volume":"164","author":"Glassman","year":"2000","journal-title":"J. Urol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0125","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1053\/ajkd.2001.27720","article-title":"Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality","volume":"38","author":"Perrone","year":"2001","journal-title":"Am. J. Kidney Dis."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0130","doi-asserted-by":"crossref","first-page":"2048","DOI":"10.1681\/ASN.V5122048","article-title":"Causes of death in autosomal dominant polycystic kidney disease","volume":"5","author":"Fick","year":"1995","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0135","doi-asserted-by":"crossref","first-page":"1439","DOI":"10.1016\/S0140-6736(01)06531-X","article-title":"Autosomal dominant polycystic kidney disease: modification of disease progression","volume":"358","author":"Peters","year":"2001","journal-title":"Lancet"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0140","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1016\/0888-7543(95)80020-M","article-title":"Evidence for a third genetic locus for autosomal dominant polycystic kidney disease","volume":"25","author":"Daoust","year":"1995","journal-title":"Genomics"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0145","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1136\/jmg.38.4.238","article-title":"Autosomal dominant polycystic kidney disease unlinked to the PKD1 and PKD2 loci presenting as familial cerebral aneurysm","volume":"38","author":"McConnell","year":"2001","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0150","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/0140-6736(90)91870-G","article-title":"Possible locus for polycystic kidney disease on chromosome 2","volume":"336","author":"Norby","year":"1990","journal-title":"Lancet"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0155","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/S0140-6736(98)03495-3","article-title":"Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group","volume":"353","author":"Hateboer","year":"1999","journal-title":"Lancet"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0160","doi-asserted-by":"crossref","first-page":"3013","DOI":"10.1681\/ASN.2006080835","article-title":"Cyst number but not the rate of cystic growth is associated with the mutated gene in autosomal dominant polycystic kidney disease","volume":"17","author":"Harris","year":"2006","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0165","doi-asserted-by":"crossref","first-page":"2143","DOI":"10.1681\/ASN.2006121387","article-title":"Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease","volume":"18","author":"Rossetti","year":"2007","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0170","first-page":"1311","article-title":"The PKD1 gene product, \u201cpolycystin-1,\u201d is a tyrosine-phosphorylated protein that colocalizes with alpha2beta1-integrin in focal clusters in adherent renal epithelia","volume":"79","author":"Wilson","year":"1999","journal-title":"Lab. Invest."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0175","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1172\/JCI21753","article-title":"Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus","volume":"114","author":"Chauvet","year":"2004","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0180","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.devcel.2005.12.005","article-title":"Polycystin-1, STAT6, and P100 function in a pathway that transduces ciliary mechanosensation and is activated in polycystic kidney disease","volume":"10","author":"Low","year":"2006","journal-title":"Dev. Cell"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0185","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1042\/BST0350124","article-title":"TRP channels and kidney disease: lessons from polycystic kidney disease","volume":"35","author":"Qamar","year":"2007","journal-title":"Biochem. Soc. Trans."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0190","doi-asserted-by":"crossref","first-page":"1202","DOI":"10.1038\/ncb1326","article-title":"Polycystin-1 and polycystin-2 regulate the cell cycle through the helix\u2013loop\u2013helix inhibitor Id2","volume":"7","author":"Li","year":"2005","journal-title":"Nat. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0195","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1097\/GIM.0b013e3181a02882","article-title":"The nonmotile ciliopathies","volume":"11","author":"Tobin","year":"2009","journal-title":"Genet. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0200","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1172\/JCI37041","article-title":"Mechanistic insights into Bardet\u2013Biedl syndrome, a model ciliopathy","volume":"119","author":"Zaghloul","year":"2009","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0205","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1016\/S0092-8674(00)81793-6","article-title":"The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I","volume":"87","author":"Qian","year":"1996","journal-title":"Cell"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0210","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1038\/ng1097-179","article-title":"Perinatal lethality with kidney and pancreas defects in mice with a targetted Pkd1 mutation","volume":"17","author":"Lu","year":"1997","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0215","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1038\/5944","article-title":"Late onset of renal and hepatic cysts in Pkd1-targeted heterozygotes","volume":"21","author":"Lu","year":"1999","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0220","doi-asserted-by":"crossref","first-page":"3069","DOI":"10.1093\/hmg\/ddh336","article-title":"Lowering of Pkd1 expression is sufficient to cause polycystic kidney disease","volume":"13","author":"Lantinga-van Leeuwen","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0225","doi-asserted-by":"crossref","first-page":"1174","DOI":"10.1093\/hmg\/ddp588","article-title":"Pkd1 transgenic mice: adult model of polycystic kidney disease with extrarenal and renal phenotypes","volume":"19","author":"Kurbegovic","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0230","doi-asserted-by":"crossref","first-page":"1490","DOI":"10.1038\/nm1675","article-title":"A critical developmental switch defines the kinetics of kidney cyst formation after loss of Pkd1","volume":"13","author":"Piontek","year":"2007","journal-title":"Nat. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0235","doi-asserted-by":"crossref","first-page":"3188","DOI":"10.1093\/hmg\/ddm299","article-title":"Kidney-specific inactivation of the Pkd1 gene induces rapid cyst formation in developing kidneys and a slow onset of disease in adult mice","volume":"16","author":"Lantinga-van Leeuwen","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0240","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1093\/hmg\/9.3.447","article-title":"Genetic evidence for a trans-heterozygous model for cystogenesis in autosomal dominant polycystic kidney disease","volume":"9","author":"Koptides","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0245","doi-asserted-by":"crossref","first-page":"2523","DOI":"10.1093\/hmg\/ddp147","article-title":"Renal injury is a third hit promoting rapid development of adult polycystic kidney disease","volume":"18","author":"Takakura","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0250","doi-asserted-by":"crossref","first-page":"37285","DOI":"10.1074\/jbc.M704562200","article-title":"Heat shock transcription factor 1 is required for maintenance of ciliary beating in mice","volume":"282","author":"Takaki","year":"2007","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0255","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1016\/j.resp.2008.05.010","article-title":"Efficient mucociliary transport relies on efficient regulation of ciliary beating","volume":"163","author":"Braiman","year":"2008","journal-title":"Respir. Physiol. Neurobiol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0260","doi-asserted-by":"crossref","first-page":"2133","DOI":"10.1093\/hmg\/ddh219","article-title":"Dysfunction of axonemal dynein heavy chain Mdnah5 inhibits ependymal flow and reveals a novel mechanism for hydrocephalus formation","volume":"13","author":"Ibanez-Tallon","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0265","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1083\/jcb.200710162","article-title":"Mutations in Hydin impair ciliary motility in mice","volume":"180","author":"Lechtreck","year":"2008","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0270","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1126\/science.1124534","article-title":"The primary cilium as the cell's antenna: signaling at a sensory organelle","volume":"313","author":"Singla","year":"2006","journal-title":"Science"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0275","doi-asserted-by":"crossref","first-page":"552","DOI":"10.1007\/BF02975837","article-title":"Beitr\u00e4ge zur Kenntnis einiger Dr\u00fcsen und Epithelien","volume":"52","author":"Zimmermann","year":"1898","journal-title":"Arch. mikr. Anat."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0280","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.devcel.2006.06.009","article-title":"The emerging complexity of the vertebrate cilium: new functional roles for an ancient organelle","volume":"11","author":"Davis","year":"2006","journal-title":"Dev. Cell"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0285","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1242\/jcs.061093","article-title":"The perennial organelle: assembly and disassembly of the primary cilium","volume":"123","author":"Seeley","year":"2010","journal-title":"J. Cell Sci."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0290","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1002\/ajmg.c.30227","article-title":"Ciliary biology: understanding the cellular and genetic basis of human ciliopathies","volume":"151C","author":"Cardenas-Rodriguez","year":"2009","journal-title":"Am. J. Med. Genet. C Semin. Med. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0295","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1002\/ajmg.c.30231","article-title":"Making sense of cilia in disease: the human ciliopathies","volume":"151C","author":"Baker","year":"2009","journal-title":"Am. J. Med. Genet. C Semin. Med. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0300","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1083\/jcb.200503053","article-title":"Cilia and the cell cycle?","volume":"169","author":"Quarmby","year":"2005","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0305","doi-asserted-by":"crossref","first-page":"1255","DOI":"10.1016\/j.cell.2007.06.018","article-title":"The primary cilium: keeper of the key to cell division","volume":"129","author":"Pan","year":"2007","journal-title":"Cell"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0310","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1083\/jcb.43.2.361","article-title":"The relation between cilia and mitoses in the mouse adenohypophysis","volume":"43","author":"Dingemans","year":"1969","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0315","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1083\/jcb.49.1.226","article-title":"The relationship of cilia with cell division and differentiation","volume":"49","author":"Fonte","year":"1971","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0320","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1083\/jcb.15.2.363","article-title":"Centrioles and the formation of rudimentary cilia by fibroblasts and smooth muscle cells","volume":"15","author":"Sorokin","year":"1962","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0325","doi-asserted-by":"crossref","first-page":"1381","DOI":"10.1681\/ASN.2006111215","article-title":"Role of primary cilia in the pathogenesis of polycystic kidney disease","volume":"18","author":"Yoder","year":"2007","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0330","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1016\/j.ceb.2010.03.010","article-title":"The ciliary membrane","volume":"22","author":"Rohatgi","year":"2010","journal-title":"Curr. Opin. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0335","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/S0070-2153(08)00802-8","article-title":"Intraflagellar transport (IFT) role in ciliary assembly, resorption and signalling","volume":"85","author":"Pedersen","year":"2008","journal-title":"Curr. Top. Dev. Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0340","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.devcel.2007.08.007","article-title":"Centrioles want to move out and make cilia","volume":"13","author":"Pearson","year":"2007","journal-title":"Dev. Cell"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0345","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1083\/jcb.149.2.317","article-title":"The respective contributions of the mother and daughter centrioles to centrosome activity and behavior in vertebrate cells","volume":"149","author":"Piel","year":"2000","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0350","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1083\/jcb.54.2.246","article-title":"The three-dimensional structure of the basal body from the rhesus monkey oviduct","volume":"54","author":"Anderson","year":"1972","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0355","doi-asserted-by":"crossref","first-page":"1586","DOI":"10.1016\/S0960-9822(01)00484-5","article-title":"Localization of intraflagellar transport protein IFT52 identifies basal body transitional fibers as the docking site for IFT particles","volume":"11","author":"Deane","year":"2001","journal-title":"Curr. Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0360","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1095\/biolreprod10.4.391","article-title":"Influence of ovarian hormones on formation of solitary cilia and behavior of the centrioles in uterine epithelial cells of the rat","volume":"10","author":"Tachi","year":"1974","journal-title":"Biol. Reprod."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0365","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1038\/nrm952","article-title":"Intraflagellar transport","volume":"3","author":"Rosenbaum","year":"2002","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0370","doi-asserted-by":"crossref","first-page":"5519","DOI":"10.1073\/pnas.90.12.5519","article-title":"A motility in the eukaryotic flagellum unrelated to flagellar beating","volume":"90","author":"Kozminski","year":"1993","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0375","doi-asserted-by":"crossref","first-page":"4457","DOI":"10.1073\/pnas.94.9.4457","article-title":"Transport of a novel complex in the cytoplasmic matrix of Chlamydomonas flagella","volume":"94","author":"Piperno","year":"1997","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0380","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1083\/jcb.119.6.1605","article-title":"Polarity of flagellar assembly in Chlamydomonas","volume":"119","author":"Johnson","year":"1992","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0385","doi-asserted-by":"crossref","first-page":"5286","DOI":"10.1073\/pnas.0836980100","article-title":"Kidney-specific inactivation of the KIF3A subunit of kinesin-II inhibits renal ciliogenesis and produces polycystic kidney disease","volume":"100","author":"Lin","year":"2003","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0390","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1146\/annurev.cellbio.19.111401.091318","article-title":"Intraflagellar transport","volume":"19","author":"Scholey","year":"2003","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0395","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/s00232-001-0075-4","article-title":"Bending the MDCK cell primary cilium increases intracellular calcium","volume":"184","author":"Praetorius","year":"2001","journal-title":"J. Membr. Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0400","first-page":"314","article-title":"Insertional mutagenesis and molecular analysis of a new gene associated with polycystic kidney disease","volume":"107","author":"Yoder","year":"1995","journal-title":"Proc. Assoc. Am. Physicians"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0405","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1083\/jcb.151.3.709","article-title":"Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagella","volume":"151","author":"Pazour","year":"2000","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0410","doi-asserted-by":"crossref","first-page":"1960","DOI":"10.1002\/dvdy.21515","article-title":"The Oak Ridge Polycystic Kidney mouse: modeling ciliopathies of mice and men","volume":"237","author":"Lehman","year":"2008","journal-title":"Dev. Dyn."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0415","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1038\/43913","article-title":"A polycystic kidney-disease gene homologue required for male mating behaviour in C. elegans","volume":"401","author":"Barr","year":"1999","journal-title":"Nature"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0420","doi-asserted-by":"crossref","first-page":"R378","DOI":"10.1016\/S0960-9822(02)00877-1","article-title":"Polycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney disease","volume":"12","author":"Pazour","year":"2002","journal-title":"Curr. Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0425","doi-asserted-by":"crossref","first-page":"2508","DOI":"10.1097\/01.ASN.0000029587.47950.25","article-title":"The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia","volume":"13","author":"Yoder","year":"2002","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0430","doi-asserted-by":"crossref","first-page":"2703","DOI":"10.1093\/hmg\/ddg274","article-title":"Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia","volume":"12","author":"Ward","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0435","doi-asserted-by":"crossref","first-page":"1855","DOI":"10.1681\/ASN.2006121344","article-title":"Nephronophthisis-associated ciliopathies","volume":"18","author":"Hildebrandt","year":"2007","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0440","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1038\/ng1217","article-title":"Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination","volume":"34","author":"Otto","year":"2003","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0445","first-page":"F132","article-title":"Analysis and modeling of the primary cilium bending response to fluid shear","volume":"272","author":"Schwartz","year":"1997","journal-title":"Am. J. Physiol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0450","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1007\/s00232-002-1042-4","article-title":"Removal of the MDCK cell primary cilium abolishes flow sensing","volume":"191","author":"Praetorius","year":"2003","journal-title":"J. Membr. Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0455","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1038\/ng1076","article-title":"Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells","volume":"33","author":"Nauli","year":"2003","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0460","doi-asserted-by":"crossref","first-page":"3241","DOI":"10.1128\/MCB.00072-07","article-title":"Fibrocystin\/polyductin, found in the same protein complex with polycystin-2, regulates calcium responses in kidney epithelia","volume":"27","author":"Wang","year":"2007","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0465","doi-asserted-by":"crossref","first-page":"1161","DOI":"10.1161\/CIRCULATIONAHA.107.710111","article-title":"Endothelial cilia are fluid shear sensors that regulate calcium signaling and nitric oxide production through polycystin-1","volume":"117","author":"Nauli","year":"2008","journal-title":"Circulation"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0470","doi-asserted-by":"crossref","first-page":"F930","DOI":"10.1152\/ajprenal.00285.2006","article-title":"Human ADPKD primary cyst epithelial cells with a novel, single codon deletion in the PKD1 gene exhibit defective ciliary polycystin localization and loss of flow-induced Ca2+ signaling","volume":"292","author":"Xu","year":"2007","journal-title":"Am. J. Physiol. Ren. Physiol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0475","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/S0092-8674(02)00716-X","article-title":"PKD1 induces p21(waf1) and regulation of the cell cycle via direct activation of the JAK\u2013STAT signaling pathway in a process requiring PKD2","volume":"109","author":"Bhunia","year":"2002","journal-title":"Cell"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0480","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1172\/JCI22850","article-title":"Pkd1 regulates immortalized proliferation of renal tubular epithelial cells through p53 induction and JNK activation","volume":"115","author":"Nishio","year":"2005","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0485","doi-asserted-by":"crossref","first-page":"5466","DOI":"10.1073\/pnas.0509694103","article-title":"The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease","volume":"103","author":"Shillingford","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0490","doi-asserted-by":"crossref","first-page":"55455","DOI":"10.1074\/jbc.M402905200","article-title":"Polycystin-1 activates the calcineurin\/NFAT (nuclear factor of activated T-cells) signaling pathway","volume":"279","author":"Puri","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0495","doi-asserted-by":"crossref","first-page":"4947","DOI":"10.1074\/jbc.274.8.4947","article-title":"The polycystic kidney disease 1 gene product modulates Wnt signaling","volume":"274","author":"Kim","year":"1999","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0500","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1146\/annurev.cellbio.23.090506.123249","article-title":"Cilia and developmental signaling","volume":"23","author":"Eggenschwiler","year":"2007","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0505","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1126\/science.1146180","article-title":"Cell signaling. A ciliary signaling switch","volume":"317","author":"Christensen","year":"2007","journal-title":"Science"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0510","doi-asserted-by":"crossref","first-page":"3938","DOI":"10.1074\/jbc.M410697200","article-title":"Cystic renal neoplasia following conditional inactivation of apc in mouse renal tubular epithelium","volume":"280","author":"Qian","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0515","doi-asserted-by":"crossref","first-page":"1010","DOI":"10.1038\/ng.179","article-title":"Loss of Fat4 disrupts PCP signaling and oriented cell division and leads to cystic kidney disease","volume":"40","author":"Saburi","year":"2008","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0520","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1038\/ncb1670","article-title":"Kif3a constrains beta-catenin-dependent Wnt signalling through dual ciliary and non-ciliary mechanisms","volume":"10","author":"Corbit","year":"2008","journal-title":"Nat. Cell Biol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0525","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1159\/000208212","article-title":"Primary cilia and signaling pathways in mammalian development, health and disease","volume":"111","author":"Veland","year":"2009","journal-title":"Nephron Physiol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0530","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1016\/S0272-6386(96)90378-9","article-title":"The etiology, pathogenesis, and treatment of autosomal dominant polycystic kidney disease: recent advances","volume":"28","author":"Grantham","year":"1996","journal-title":"Am. J. Kidney Dis."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0535","doi-asserted-by":"crossref","first-page":"1234","DOI":"10.1111\/j.1523-1755.2005.00201.x","article-title":"Molecular pathogenesis of ADPKD: the polycystin complex gets complex","volume":"67","author":"Ong","year":"2005","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0540","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1681\/ASN.2005050534","article-title":"Polycystin-1 induces resistance to apoptosis through the phosphatidylinositol 3-kinase\/Akt signaling pathway","volume":"17","author":"Boca","year":"2006","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0545","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1111\/j.1440-1797.2007.00869.x","article-title":"Polycystic kidney disease and the renal cilium","volume":"12","author":"Deane","year":"2007","journal-title":"Nephrology (Carlton)"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0550","doi-asserted-by":"crossref","first-page":"2719","DOI":"10.1681\/ASN.2005090979","article-title":"A truncated polycystin-2 protein causes polycystic kidney disease and retinal degeneration in transgenic rats","volume":"17","author":"Gallagher","year":"2006","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0555","doi-asserted-by":"crossref","first-page":"964","DOI":"10.1111\/j.1523-1755.2004.00843.x","article-title":"Cyclic AMP promotes growth and secretion in human polycystic kidney epithelial cells","volume":"66","author":"Belibi","year":"2004","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0560","doi-asserted-by":"crossref","first-page":"1421","DOI":"10.1681\/ASN.2009050501","article-title":"Type II calcimimetics and polycystic kidney disease: unanswered questions","volume":"20","author":"Torres","year":"2009","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0565","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1038\/ki.1991.239","article-title":"C-erb B-2 amplification in cystic renal disease","volume":"40","author":"Herrera","year":"1991","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0570","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1074\/jbc.M507845200","article-title":"Polycystin-2 regulates proliferation and branching morphogenesis in kidney epithelial cells","volume":"281","author":"Grimm","year":"2006","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0575","doi-asserted-by":"crossref","first-page":"5247","DOI":"10.1073\/pnas.0601352103","article-title":"mTOR is out of control in polycystic kidney disease","volume":"103","author":"Mostov","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0580","doi-asserted-by":"crossref","first-page":"776","DOI":"10.4161\/cc.6.7.4047","article-title":"Targeting dysregulated cell cycle and apoptosis for polycystic kidney disease therapy","volume":"6","author":"Ibraghimov-Beskrovnaya","year":"2007","journal-title":"Cell Cycle"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0585","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1038\/ki.1996.304","article-title":"The cystic fibrosis transmembrane conductance regulator mediates transepithelial fluid secretion by human autosomal dominant polycystic kidney disease epithelium in vitro","volume":"50","author":"Davidow","year":"1996","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0590","doi-asserted-by":"crossref","first-page":"1242","DOI":"10.1681\/ASN.V641242","article-title":"Evidence for a potent lipid secretagogue in the cyst fluids of patients with autosomal dominant polycystic kidney disease","volume":"6","author":"Grantham","year":"1995","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0595","doi-asserted-by":"crossref","first-page":"2619","DOI":"10.1097\/01.ASN.0000034220.09301.9C","article-title":"Sulfonylurea-sensitive K(+) transport is involved in Cl(\u2212) secretion and cyst trowth by cultured ADPKD cells","volume":"13","author":"Sullivan","year":"2002","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0600","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1038\/ki.2008.246","article-title":"KCa3.1 potassium channels are critical for cAMP-dependent chloride secretion and cyst growth in autosomal-dominant polycystic kidney disease","volume":"74","author":"Albaqumi","year":"2008","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0605","first-page":"529","article-title":"Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis","volume":"19","author":"Xu","year":"2006","journal-title":"J. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0610","doi-asserted-by":"crossref","first-page":"3424","DOI":"10.1681\/ASN.2006030295","article-title":"Early embryonic renal tubules of wild-type and polycystic kidney disease kidneys respond to cAMP stimulation with cystic fibrosis transmembrane conductance regulator\/Na(+), K(+), 2Cl(\u2212) Co-transporter-dependent cystic dilation","volume":"17","author":"Magenheimer","year":"2006","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0615","doi-asserted-by":"crossref","first-page":"1926","DOI":"10.1111\/j.1523-1755.2004.00967.x","article-title":"The relationship between cell proliferation, Cl-secretion, and renal cyst growth: a study using CFTR inhibitors","volume":"66","author":"Li","year":"2004","journal-title":"Kidney Int."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0620","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1016\/0092-8674(91)90072-7","article-title":"Nucleoside triphosphates are required to open the CFTR chloride channel","volume":"67","author":"Anderson","year":"1991","journal-title":"Cell"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0625","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1681\/ASN.2006111218","article-title":"Identification of a forskolin-like molecule in human renal cysts","volume":"18","author":"Putnam","year":"2007","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0630","doi-asserted-by":"crossref","first-page":"984","DOI":"10.1681\/ASN.V34984","article-title":"Cyst fluid from human autosomal dominant polycystic kidneys promotes cyst formation and expansion by renal epithelial cells in vitro","volume":"3","author":"Ye","year":"1992","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0635","doi-asserted-by":"crossref","first-page":"1323","DOI":"10.1038\/nm935","article-title":"Inhibition of renal cystic disease development and progression by a vasopressin V2 receptor antagonist","volume":"9","author":"Gattone","year":"2003","journal-title":"Nat. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0640","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1038\/nm1004","article-title":"Effective treatment of an orthologous model of autosomal dominant polycystic kidney disease","volume":"10","author":"Torres","year":"2004","journal-title":"Nat. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0645","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1016\/S0272-6386(97)90496-0","article-title":"Renal accumulation and excretion of cyclic adenosine monophosphate in a murine model of slowly progressive polycystic kidney disease","volume":"30","author":"Yamaguchi","year":"1997","journal-title":"Am. J. Kidney Dis."},{"issue":"Pt 2","key":"10.1016\/j.bbadis.2010.11.014_bb0650","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1042\/bj3510289","article-title":"Meaningful relationships: the regulation of the Ras\/Raf\/MEK\/ERK pathway by protein interactions","volume":"351","author":"Kolch","year":"2000","journal-title":"Biochem. J."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0655","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1111\/j.0954-6820.1986.tb03330.x","article-title":"Expansion of extracellular volume in early polycystic kidney disease","volume":"219","author":"Danielsen","year":"1986","journal-title":"Acta Med. Scand."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0660","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1002\/(SICI)1520-6408(1999)24:3\/4<309::AID-DVG14>3.0.CO;2-5","article-title":"Developmental expression of urine concentration-associated genes and their altered expression in murine infantile-type polycystic kidney disease","volume":"24","author":"Gattone","year":"1999","journal-title":"Dev. Genet."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0665","doi-asserted-by":"crossref","first-page":"1052","DOI":"10.1681\/ASN.2009121291","article-title":"Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease","volume":"21","author":"Hogan","year":"2010","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0670","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/j.1527-3466.2007.00001.x","article-title":"Tolvaptan, an orally active vasopressin V(2)-receptor antagonist \u2014 pharmacology and clinical trials","volume":"25","author":"Miyazaki","year":"2007","journal-title":"Cardiovasc. Drug Rev."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0675","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1097\/MNH.0b013e3283262ab0","article-title":"Advances in the pathogenesis and treatment of polycystic kidney disease","volume":"18","author":"Patel","year":"2009","journal-title":"Curr. Opin. Nephrol. Hypertens."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0680","doi-asserted-by":"crossref","first-page":"1300","DOI":"10.1681\/ASN.2007070828","article-title":"Small-molecule CFTR inhibitors slow cyst growth in polycystic kidney disease","volume":"19","author":"Yang","year":"2008","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0685","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.2165\/00003495-200464120-00002","article-title":"Recent advances in understanding the pathogenesis of polycystic kidney disease: therapeutic implications","volume":"64","author":"Cowley","year":"2004","journal-title":"Drugs"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0690","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1016\/j.coph.2005.04.016","article-title":"Emerging therapies for polycystic kidney disease","volume":"5","author":"Gattone","year":"2005","journal-title":"Curr. Opin. Pharmacol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0695","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1038\/nature05348","article-title":"Long-lasting arrest of murine polycystic kidney disease with CDK inhibitor roscovitine","volume":"444","author":"Bukanov","year":"2006","journal-title":"Nature"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0700","doi-asserted-by":"crossref","first-page":"7214","DOI":"10.1074\/jbc.M805890200","article-title":"Cyst formation in kidney via B-Raf signaling in the PKD2 transgenic mice","volume":"284","author":"Park","year":"2009","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0705","doi-asserted-by":"crossref","first-page":"1604","DOI":"10.1681\/ASN.2004090800","article-title":"Extracellular signal-regulated kinase inhibition slows disease progression in mice with polycystic kidney disease","volume":"17","author":"Omori","year":"2006","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0710","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1093\/ndt\/gfi181","article-title":"Inhibition of mTOR with sirolimus slows disease progression in Han:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD)","volume":"21","author":"Wahl","year":"2006","journal-title":"Nephrol. Dial. Transplant."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0715","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1074\/jbc.270.2.815","article-title":"Isolation of a protein target of the FKBP12-rapamycin complex in mammalian cells","volume":"270","author":"Sabers","year":"1995","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0720","doi-asserted-by":"crossref","first-page":"3271","DOI":"10.1101\/gad.13.24.3271","article-title":"The TOR signaling cascade regulates gene expression in response to nutrients","volume":"13","author":"Cardenas","year":"1999","journal-title":"Genes Dev."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0725","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1681\/ASN.2009040421","article-title":"Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1","volume":"21","author":"Shillingford","year":"2009","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0730","doi-asserted-by":"crossref","first-page":"1312","DOI":"10.2215\/CJN.01360210","article-title":"Prospects for mTOR inhibitor use in patients with polycystic kidney disease and hamartomatous diseases","volume":"5","author":"Torres","year":"2010","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0735","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1056\/NEJMoa0907419","article-title":"Sirolimus and kidney growth in autosomal dominant polycystic kidney disease","volume":"363","author":"Serra","year":"2010","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0740","doi-asserted-by":"crossref","first-page":"830","DOI":"10.1056\/NEJMoa1003491","article-title":"Everolimus in patients with autosomal dominant polycystic kidney disease","volume":"363","author":"Walz","year":"2010","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0745","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1038\/nm1783","article-title":"A tumor necrosis factor-alpha-mediated pathway promoting autosomal dominant polycystic kidney disease","volume":"14","author":"Li","year":"2008","journal-title":"Nat. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0750","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1038\/sj.bjc.6603509","article-title":"A phase I trial of the selective oral cyclin-dependent kinase inhibitor seliciclib (CYC202; R-Roscovitine), administered twice daily for 7days every 21days","volume":"96","author":"Benson","year":"2007","journal-title":"Br. J. Cancer"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0755","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1038\/nm.2171","article-title":"Inhibition of glucosylceramide accumulation results in effective blockade of polycystic kidney disease in mouse models","volume":"16","author":"Natoli","year":"2010","journal-title":"Nat. Med."},{"key":"10.1016\/j.bbadis.2010.11.014_bb0760","doi-asserted-by":"crossref","first-page":"21819","DOI":"10.1073\/pnas.0911987106","article-title":"Chemical modifier screen identifies HDAC inhibitors as suppressors of PKD models","volume":"106","author":"Cao","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.bbadis.2010.11.014_bb0765","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1242\/dev.049437","article-title":"Polycystin-dependent fluid flow sensing targets histone deacetylase 5 to prevent the development of renal cysts","volume":"137","author":"Xia","year":"2010","journal-title":"Development"}],"container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0925443910002693?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0925443910002693?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T04:39:40Z","timestamp":1760416780000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0925443910002693"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,10]]},"references-count":153,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2011,10]]}},"alternative-id":["S0925443910002693"],"URL":"https:\/\/doi.org\/10.1016\/j.bbadis.2010.11.014","relation":{},"ISSN":["0925-4439"],"issn-type":[{"value":"0925-4439","type":"print"}],"subject":[],"published":{"date-parts":[[2011,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Polycystic kidney disease: Pathogenesis and potential therapies","name":"articletitle","label":"Article Title"},{"value":"Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.bbadis.2010.11.014","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2010 Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}